OBJECTIVES

To describe the clinical profile and outcomes of a series of adult patients diagnosed with ocular myasthenia gravis and to evaluate the potential factors affecting the risk of generalization.

This retrospective cohort study involved a medical chart review of adult patients seen from 2012 to 2019 at the neuro-ophthalmology clinic of a tertiary Philippine hospital with a clinical diagnosis of ocular myasthenia gravis supported by serologic, electrophysiologic, or pharmacologic test results. Outcomes of interest were complete stable remission, pharmacologic remission, minimal manifestations, and generalization. Kaplan-Meier method and log-rank test were used to analyze the probability of generalization.

The study sample consisted of 16 patients. The female to male ratio was 3:1. Mean age at symptom onset was 39 years. All patients received pharmacologic treatment, while two patients underwent thymectomy. No patient had remission as of last follow-up. Three patients had conversion of ocular myasthenia gravis to generalized myasthenia gravis. Mean time from symptom onset to generalization was 10.7 months. The generalization curves of patients who were symptomatic for less than two years and those who were symptomatic for at least two years prior to consult were significantly different (p = 0.049).

In this single-center study, there was female predominance among adult patients diagnosed with ocular myasthenia gravis. The incidence of generalization was 4 per 100 person-years while the 2-year probability of generalization was 30%. Further study is needed in order to determine the factors affecting the risk of generalization.

BACKGROUND: Neuromuscular blocking agents (NMBAs) and neuromuscular monitoring in anesthetic management are integral for endotracheal intubation, better visualization of the surgical field, and prevention of residual neuromuscular blockade and pulmonary complications. Sugammadex is a drug that reduces risk of residual neuromuscular blockade, with more rapid recovery compared to anticholinesterase. The purpose of this study was to investigate current usage status of NMBAs and antagonist with neuromuscular monitoring, among anesthesiologists in Korea.METHODS: Anesthesiologists working in Korea were invited to participate in an online survey via email January 2–February 28, 2018. The questionnaire consisted of 45 items, including preferred NMBAs, antagonists, neuromuscular monitoring, and complications related to the use sugammadex. A total of 174 responses were analyzed.RESULTS: Rocuronium was a commonly used NMBA for endotracheal intubation (98%) of hospitals, and maintenance of anesthesia (83.3%) in of hospitals. Sugammadex, pyridostigmine, and neostigmine were used in 89.1%, 87.9%, and 45.4% of hospitals. Neuromuscular monitoring was employed in 79.3% of hospitals; however only 39.7% of hospitals used neuromuscular monitoring before antagonist administration. Usual dosage range of sugammadex was 2.1–4 mg/kg in 35.1% of hospitals, within 2 mg/kg in 34.5% of hospitals, and 1 vial regardless of body weight in 22.4% of hospitals. Sugammadex-related complications were encountered by 14.9% of respondents.CONCLUSIONS: This survey indicates several minor problems associated with the use of antagonists and neuromuscular monitoring. However, most anesthesiologists appear to have appropriate information regarding the usage of NMBAs and sugammadex.
Anesthesia ; Body Weight ; Delayed Emergence from Anesthesia ; Electronic Mail ; Intubation, Intratracheal ; Korea ; Neostigmine ; Neuromuscular Blockade ; Neuromuscular Blocking Agents ; Neuromuscular Monitoring ; Pyridostigmine Bromide ; Surveys and Questionnaires

BACKGROUND: Sugammadex reverses rocuronium-induced neuromuscular blockade quickly and effectively. Herein, we compared the efficacy of sugammadex and pyridostigmine in the reversal of rocuronium-induced light block or minimal block in pediatric patients scheduled for elective entropion surgery. METHODS: A prospective randomized study was conducted in 60 pediatric patients aged 2–11 years who were scheduled for entropion surgery under sevoflurane anesthesia. Neuromuscular blockade was achieved by administration of 0.6 mg/kg rocuronium and assessed using the train-of-four (TOF) technique. Patients were randomly assigned to 2 groups receiving either sugammadex 2 mg/kg or pyridostigmine 0.2 mg/kg and glycopyrrolate 0.01 mg/kg at the end of surgery. Primary outcomes were time from administration of reversal agents to TOF ratio 0.9 and TOF ratio 1.0. Time from the administration of reversal agents to extubation and postoperative adverse events were also recorded. RESULTS: There were no significant differences in the demographic variables. Time from the administration of reversal agents to TOF ratio 0.9 and TOF ratio 1.0 were significantly shorter in the sugammadex group than in the pyridostigmine plus glycopyrrolate group: 1.30 ± 0.84 vs. 3.53 ± 2.73 min (P < 0.001) and 2.75 ± 1.00 vs. 5.73 ± 2.83 min (P < 0.001), respectively. Extubation time was shorter in the sugammadex group. Adverse events, such as skin rash, nausea, vomiting, and postoperative residual neuromuscular blockade (airway obstruction), were not statistically different between the two groups. CONCLUSIONS: Sugammadex provided more rapid reversal of rocuronium-induced neuromuscular blockade in pediatric patients undergoing surgery than did pyridostigmine plus glycopyrrolate.
Anesthesia ; Delayed Emergence from Anesthesia ; Entropion ; Exanthema ; Glycopyrrolate ; Humans ; Nausea ; Neuromuscular Blockade ; Neuromuscular Monitoring ; Pediatrics ; Prospective Studies ; Pyridostigmine Bromide ; Vomiting

Pelizaeus-Merzbacher disease (PMD) is a progressive and degenerative chromosomal disorder of the central nervous system caused by defective myelin production. Few case reports have been issued on the anesthetic management of PMD, because of its extremely low incidence. We anesthetized a 13-year-old female patient diagnosed with PMD for ophthalmic surgery because of intermittent exotropia. General anesthesia was induced and maintained with propofol and sevoflurane in air and oxygen. Rocuronium was administered to facilitate orotracheal intubation, and residual neuromuscular blockage was reversed with pyridostigmine. Between emergence to 24 hours postoperatively, her muscle power completely recovered and no unpredictable events occurred. Summarizing, anesthesiologists should be concerned about the high possibility of aspiration, spasticity, and seizure during the perioperative period in patients with even mild PMD. Appropriate preoperative evaluation, intraoperative monitoring, and choice of proper anesthetic drugs enable safe anesthesia in patients with PMD.
Adolescent* ; Anesthesia ; Anesthesia, General* ; Anesthetics ; Central Nervous System ; Chromosome Disorders ; Exotropia ; Female ; Humans ; Incidence ; Intubation ; Monitoring, Intraoperative ; Muscle Spasticity ; Myelin Sheath ; Oxygen ; Pelizaeus-Merzbacher Disease* ; Perioperative Period ; Propofol ; Pyridostigmine Bromide ; Seizures

Pediatric chronic intestinal pseudo-obstruction is a rare disorder characterized by a severe impairment of gastrointestinal motility leading to intestinal obstruction symptoms in the absence of mechanical causes. The diagnosis is usually clinical and diagnostic work is usually aimed to rule out mechanical obstruction and to identify any underlying diseases. Treatment is challenging and requires a multidisciplinary effort. In this manuscript we describe the youngest child successfully treated with the orally administrable, long-acting, reversible anti-cholinesterase drug, pyridostigmine. Like other drugs belonging to cholinesterase inhibitors, pyridostigmine enhances gut motility by increasing acetylcholine availability in the enteric nervous system and neuro-muscular junctions. Based on the direct evidence from the reported case, we reviewed the current literature on the use of pyridostigmine in severe pediatric dysmotility focusing on intestinal pseudo-obstruction. The overall data emerged from the few published studies suggest that pyridostigmine is an effective and usually well tolerated therapeutic options for patients with intestinal pseudo-obstruction. More specifically, the main results obtained by pyridostigmine included marked reduction of abdominal distension, reduced need of parenteral nutrition, and improvement of oral feeding. The present case and review on pyridostigmine pave the way for eagerly awaited future randomized controlled studies testing the efficacy of cholinesterase inhibitors in pediatric severe gut dysmotility.
Acetylcholine ; Child ; Cholinesterase Inhibitors ; Diagnosis ; Enteric Nervous System ; Female ; Gastrointestinal Motility ; Humans ; Intestinal Obstruction ; Intestinal Pseudo-Obstruction ; Parenteral Nutrition ; Pyridostigmine Bromide

Dermatomyositis is an idiopathic inflammatory myopathy characterized by skin changes and muscle weakness. Depending on the involvement of various muscles, dermatomyositis can cause aspiration pneumonia, ventilatory impairment, and heart failure. Several reports have documented normal or prolonged neuromuscular blockade following administration of different non-depolarizing neuromuscular blockers in patients with dermatomyositis. We observed delayed onset of blockade and prolonged recovery following administration of 0.6 mg/kg rocuronium in a patient with dermatomyositis. However, when the train-of-four ratio reached 0.3, the patient was administered pyridostigmine and glycopyrrolate, which led to normal response to reversal of rocuronium. The patient was extubated without respiratory complications. The outcomes of this case indicate that response to the usual dosage of muscle relaxants in patients with dermatomyositis might be different from that in patients without this condition. Anesthesiologists should pay attention to preoperative cardiorespiratory evaluation and intraoperative neuromuscular monitoring.
Anesthesia, General ; Dermatomyositis ; Glycopyrrolate ; Heart Failure ; Humans ; Muscle Weakness ; Muscles ; Myositis ; Neuromuscular Blockade ; Neuromuscular Blocking Agents ; Neuromuscular Monitoring ; Pneumonia, Aspiration ; Pyridostigmine Bromide ; Skin

BACKGROUND: This study aimed to retrospectively evaluate the use of sugammadex in patients undergoing video-assisted thoracoscopic surgery (VATS) lobectomy. METHODS: Data were obtained from medical record review of patients who underwent VATS lobectomy from January 2013 to November 2014. Fifty patients were divided into two groups: the sugammadex group (group S, n = 19) was administered sugammadex 2 mg/kg, while the pyridostigmine group (group P, n = 31) received pyridostigmine 20 mg with glycopyrrolate 0.2 mg or atropine 0.5 mg. The primary endpoint measure was the overall incidence of postoperative pulmonary complications including prolonged air leak, pneumonia, and atelectasis. The secondary endpoint measures were the length of postoperative hospital stay and duration of chest tube insertion. RESULTS: The overall incidence of postoperative pulmonary complications in patients in group S was significantly lower compared with that of group P (5 [26.3%] vs. 17 [54.8%]; P = 0.049). Also, the durations of chest tube insertion (5.0 [4.0–7.0] vs. 7.0 [6.0–8.0] days; P = 0.014) and postoperative hospital stay (8.0 [8.0–10.0] vs. 10.0 [9.0–11.0] days; P = 0.019) were shorter in group S compared with group P. Administration of sugammadex was associated reduced with postoperative pulmonary complications (OR: 0.22; 95% CI: 0.05–0.87; P = 0.031). CONCLUSIONS: The use of sugammadex, compared with pyridostigmine, showed a significantly reduced overall incidence of postoperative pulmonary complications and decreased duration of chest tube use and postoperative hospital stay in patients undergoing VATS lobectomy, suggesting that sugammadex might be helpful in improving clinical outcomes in such patients.
Atropine ; Chest Tubes ; Glycopyrrolate ; Humans ; Incidence ; Length of Stay ; Lung Neoplasms* ; Lung* ; Medical Records ; Pneumonia ; Pulmonary Atelectasis ; Pyridostigmine Bromide ; Retrospective Studies ; Thoracic Surgery, Video-Assisted

In the elderly, myasthenia gravis (MG) can present with bulbar symptoms, which can be clinically difficult to diagnose from other neurological comorbid conditions. We describe a case of a 75-year-old man who had been previously diagnosed with dysphagia associated with medullary infarction but exhibited aggravation of the dysphagia later on due to a superimposed development of bulbar MG. After recovering from his initial swallowing difficulties, the patient suddenly developed ptosis, drooling, and generalized weakness with aggravated dysphagia. Two follow-up brain magnetic resonance imaging (MRI) scans displayed no new brain lesions. Antibodies to acetylcholine receptor and muscle-specific kinase were negative. Subsequent electrodiagnosis with repetitive nerve stimulation tests revealed unremarkable findings. A diagnosis of bulbar MG could only be established after fiberoptic endoscopic evaluation of swallowing (FEES) with simultaneous Tensilon application. After application of intravenous pyridostigmine, significant improvement in dysphagia and ptosis were observed both clinically and according to the FEES.
Acetylcholine ; Aged ; Antibodies ; Brain ; Deglutition Disorders ; Deglutition* ; Diagnosis ; Edrophonium* ; Electrodiagnosis ; Fees and Charges ; Follow-Up Studies ; Humans ; Infarction* ; Magnetic Resonance Imaging ; Myasthenia Gravis* ; Phosphotransferases ; Pyridostigmine Bromide ; Sialorrhea ; Stroke

Myasthenia gravis (MG) is a chronic autoimmune disease of neuromuscular blockade, characterized by muscle weakness and fatigue, and is associated with the production of autoantibodies against the skeletal muscle acetylcholine receptor, muscle-specific kinase (MuSK), low-density lipoprotein receptor-related protein 4 (LRP4), and other muscle endplate proteins. In addition, MG may be classified according the location of the affected muscles (ocular vs. generalized), patient age at symptom onset, and thymic pathology. Subgroup classification based on serum antibodies and clinical features include early-onset, late-onset, thymoma-associated, MuSK and LRP4 antibody-negative, and ocular forms of MG, and can help with therapeutic decisions and prognosis. Pyridostigmine is the chosen symptomatic treatment. For patients who do not adequately respond to symptomatic therapy, corticosteroids, other immunomodulating agents, and thymectomy are the first-line immunosuppressive treatments. The treatment of MG is highly individualized and depends on the age of the patient, the type and severity of the disease, and the pace of progression.
Acetylcholine ; Adrenal Cortex Hormones ; Adult ; Antibodies ; Autoantibodies ; Autoimmune Diseases ; Child ; Classification* ; Fatigue ; Humans ; Lipoproteins ; Muscle Weakness ; Muscle, Skeletal ; Muscles ; Myasthenia Gravis* ; Neuromuscular Blockade ; Pathology ; Phosphotransferases ; Prognosis ; Pyridostigmine Bromide ; Thymectomy

Myasthenia gravis (MG) is a chronic autoimmune disease of neuromuscular blockade, characterized by muscle weakness and fatigue, and is associated with the production of autoantibodies against the skeletal muscle acetylcholine receptor, muscle-specific kinase (MuSK), low-density lipoprotein receptor-related protein 4 (LRP4), and other muscle endplate proteins. In addition, MG may be classified according the location of the affected muscles (ocular vs. generalized), patient age at symptom onset, and thymic pathology. Subgroup classification based on serum antibodies and clinical features include early-onset, late-onset, thymoma-associated, MuSK and LRP4 antibody-negative, and ocular forms of MG, and can help with therapeutic decisions and prognosis. Pyridostigmine is the chosen symptomatic treatment. For patients who do not adequately respond to symptomatic therapy, corticosteroids, other immunomodulating agents, and thymectomy are the first-line immunosuppressive treatments. The treatment of MG is highly individualized and depends on the age of the patient, the type and severity of the disease, and the pace of progression.
Acetylcholine ; Adrenal Cortex Hormones ; Adult ; Antibodies ; Autoantibodies ; Autoimmune Diseases ; Child ; Classification* ; Fatigue ; Humans ; Lipoproteins ; Muscle Weakness ; Muscle, Skeletal ; Muscles ; Myasthenia Gravis* ; Neuromuscular Blockade ; Pathology ; Phosphotransferases ; Prognosis ; Pyridostigmine Bromide ; Thymectomy